3. INTRODUCTION
BLOOD & LUNGS 3
• The erythropoietic system plays a major role in tissue oxygenation
because the erythrocytes are the primary carriers of oxygen in the form
of oxyhemoglobin.
• Pulmonary complications are the most common cause of mortality in
patients with hematologic malignancies.
• Respiratory manifestations in these disorders may be caused by
pulmonary extension of the basic disease process, cytotoxic drug-
induced pulmonary pathology, opportunistic infections, or a combination
of these factors.
10. ANEMIA
BLOOD & LUNGS 10
• Dysnea :
- Hemoglobin concentration under 6 to 7 g/dl It is more common in acute
than in chronic anemia. Due to inadequate O2 delivery to the respiratory
muscles .
• Decreased CO transfer factor :
- The diffusing capacity decreases approximately 7% for each 1-gm
decrease in hemoglobin per 100 mL
• Auto-immune hemolytic anemia :
- Associated with: Fibrosing alveolitis , Venous thrombo-embolism ,and
Pulmonary hypertension
11. SICKLE CELL ANEMIA
BLOOD & LUNGS 11
• SCD is a serious disorder in which the body makes sickle-shaped red blood
cells. ―Sickle-shaped‖ means that the red blood cells are shaped like a
crescent.
• Sickle cells contain abnormal hemoglobin called sickle hemoglobin or
hemoglobin S. Sickle hemoglobin causes the cells to develop a sickle, or
crescent, shape.
• Sickle cells are stiff and sticky. They tend to block blood flow in the blood
vessels of the limbs and organs. Blocked blood flow can cause pain and
organ damage. It can also raise the risk for infection.
12. SICKLE CELL ANEMIA - cont
BLOOD & LUNGS 12
• 1. Pneumococcal pneumonia
- is a major cause of morbidity and mortality in children with sickle cell
disease.
- its incidence is 20 times greater than in the normal population.
- The major factors that predispose these patients to infections include:
• abnormal complement activity
• poor splenic function
• lack of type-specific pneumococcal antibody
• Local factors such as previous or concomitant pulmonary damage by vaso-
occlusion
13. SICKLE CELL ANEMIA - cont
BLOOD & LUNGS 13
• Atypical organisms, including Mycoplasma pneumoniae &
chlamydia pneumoniae have been described particularly in
children.
• upper and middle lobes being involved more often than the
others.
• Administration of pneumococcal vaccine is mandatory in
patients with sickle cell anemia.
14. SICKLE CELL ANEMIA - cont
BLOOD & LUNGS 14
• Acute chest syndrome (also called sickle chest syndrome, chest crisis,
pulmonary sickle crisis, and pulmonary infarction)
- It is seen in up to 35% of patients hospitalized with sickle cell disease.
- the leading cause of death in patients with sickle cell disease.
- It is a common manifestation of sickling-induced vasoocclusive crisis.
• A new pulmonary infiltrate involving at least one complete lung segment (not
Atelectasis), chest pain, temperature above 38.50C, tachypnea ,wheezing &
cough.
15. SICKLE CELL ANEMIA - cont
BLOOD & LUNGS 15
• Hypoxemia (PaO2 < 50 mmHg) is present in up to 40% of
patients.
• Dense bilateral lower-lung consolidations are common.
• Sickle cell crisis may be precipitated by asthmatic attacks.
• Incentive spirometry has been shown to prevent the pulmonary
complications associated with the acute chest syndrome.
16. SICKLE CELL ANEMIA - cont
BLOOD & LUNGS 16
Treatment:
• Antibiotic regimens that include coverage for CAP and atypical
pneumonia .
• Supplemental oxygen to maintain PaO2 of 70-100 mm Hg to
prevent further intravascular sickling.
• Hydration aimed at the maintenance of an euvolemic state.
• Empric anticoagulant therapy is not recommended because of
the potential risk for intracranial or renal bleeding.
17. SICKLE CELL ANEMIA - cont
BLOOD & LUNGS 17
• Treatment – cont :
• Incentive Spirometry
• Exchange transfusion in the setting of progressive pulmonary
infiltrates and hypoxemia refractory to conventional therapy.
• Inhaled nitric oxide, by reducing the adhesion of sickle
erythrocytes to the pulmonary endothelium may be effective
• Hydroxyurea decrease the number of painful crisis and episodes
of acute chest syndrome
18. SICKLE CELL ANEMIA - cont
BLOOD & LUNGS 18
• Pulmonary edema is another complication of sickle cell crisis.
• Decreased diffusing capacity of lung for carbon monoxide .
Due to loss of membrane area as a result of obstruction of
pulmonary vessels.
• chronic pulmonary disease:
- development of pulmonary hypertension & cor-pulmonale .
19. THALASSEMIA MAJOR
BLOOD & LUNGS 19
• Hemoglobin is made of two proteins: Alpha globin and beta
globin. Thalassemia occurs when there is a defect in a gene that
helps control production of one of these proteins.
• Thalassemia major is characterized by an unbalanced synthesis
of globin chain, resulting in ineffective hematopoiesis and
severe anemia.
• Patients at a young age develpe congestive cardiac failure.
20. THALASSEMIA MAJOR - cont
BLOOD & LUNGS 20
• hypoxemia in 85%,
• reduced lung volumes and flow rates in 50% and 60%, respectively,
• diminished diffusing capacity for carbon monoxide in 50%.
• Pulmonary hypertension was present in 75% (related to decreased NO
levels. Splenectomy due to development of circulating platelet thrombi ).
• right ventricular dysfunction was more prevalent than left ventricular
dysfunction
22. HEMOGLOBINOPATHIES
BLOOD & LUNGS 22
• Most patients are asymptomatic.
• Many of these hemoglobinopathies are manifested by secondary
erythrocytosis..
• Patients with hemoglobin variants with a marked increase in oxygen affinity may
demonstrate cyanosis.
• Because of the defective hemoglobin function, the arterial blood is partially
unsaturated despite normal oxygen tension, which results in elevated levels
of deoxyhemoglobin in the blood , hence the cyanosis.
• cyanosis is a cosmetic problem and warrants no specific therapy.
23. HEMOGLOBINOPATHIES
BLOOD & LUNGS 23
• HB with high O2 affinity (familial erythrocytosis) :
- Hemoglobin mutants with abnormally high oxygen affinity are
associated with secondary erythrocytosis, due to impaired
oxygen delivery to tissues.
- Pulmonary fibrosis has been described .
• Low oxygen affinity hemoglobins :
-unsaturated with oxygen that they may cause visible cyanosis.
24. METHEMOGLOBINEMIA
BLOOD & LUNGS 24
Congenital (hereditary) methemoglobinemia
• due to an altered form of hemoglobin (Hb M)
Acquired methemoglobinemia
• usually due to the ingestion of drugs or toxic substances
• .Antimalarials , Antineoplastic agents , Analgesics and
antipyretics , Herbicides and insecticides ,Metoclopramide ,.
• Antibiotics – Sulfonamides, nitrofurans, and para-aminosalicylic
acid
25. METHEMOGLOBINEMIA - cont
BLOOD & LUNGS 25
• Methemoglobinemia results when more than 1% of hemoglobin is oxidized
to ferric form.
• When hemoglobin is oxidized to methemoglobin, the heme iron becomes
Fe3+ and is incapable of binding oxygen.
• When methemoglobin level exceeds 1.5 g/dl (10% of total hemoglobin),
cyanosis becomes clinically obvious.
• Severe toxic methemoglobinemia should be treated with intravenous
methylene blue (2 mg/kg).
26. SULFHEMOGLOBINEMIA
BLOOD & LUNGS 26
Sulfaheamoglobinemia
• rare condition in which there is excess sulfhemoglobin in the
blood. which cannot be converted back to normal, functional
hemoglobin.
• It causes cyanosis even at low blood levels.
• Cyanosis occurs when there is 0.5 gm of Sulfaheamoglobin is
present .
28. COAGULOPATHIES
BLOOD & LUNGS 28
• Hemophilia and von Willebrand's disease are the most common
inherited coagulation disorders.
• Pulmonary complications such as pulmonary embolism and
alveolar hemorrhage can result from an underlying disorder of
coagulation. Both hypercoagulable and hypocoagulable states
may be associated with these complications.
29. HEMOPHILIA
BLOOD & LUNGS 29
• Hemophilia A is caused by a deficiency of factor VIII. Hemophilia
B, or Christmas disease, is due to a deficiency of factor IX .
• Pulmonary manifestations are unusual in hemophilia but
have included:
- spontaneous pneumothorax,
- hemomediastinum, tracheal obstruction by a hematoma,
- pleural hematomas
- Several cases of primary pulmonary hypertension have been described in
patients receiving factor VIII infusion.
30. DISSEMINATED INTRAVASCULAR
COAGULATION
BLOOD & LUNGS 30
• Thromboembolism and hemorrhage are the main pathologic
findings in the lungs of patients dying with DIC.
• The incidence of pulmonary hemorrhage is estimated to be
approximately 14% in DIC.
• Thrombocytopenia has been shown to lead to the development of
pulmonary hematoma, hemothorax, and fatal pulmonary
hemorrhage.
• Pulmonary infarction has been described in several patients with
DIC.
31. COAGULOPATHIES - cont
BLOOD & LUNGS 31
• Hereditary hemorrhagic telangectesia (rendo osler weber
syndrome) :
- More than 15% of patients has pulmonary A-V fistulae.
• Herman sky-pudlak syndrome :
- Occulo cutaneous albinism associated with bleeding
tendancy and abnormal pigmentation of the macrophages in the
bone marrow
- Pulmonary fibrosis especially in females.
32. THROMBOPHILIA
BLOOD & LUNGS 32
• predisposition to form
intravascular clots.
• affects the lungs through
its role in pulmonary
thromboembolism, and
pulmonary hypertension,
Inherited Thrombophilia
Factor V Leiden mutation
Prothrombin mutation
Protein S deficiency
Protein C deficiency
Antithrombin deficiency
Heparin cofactor II deficiency
Factor XII deficiency
Increased factor VIII coagulant activity
34. ANTIPHOSPHOLIPID SYNDROME AND THE
LUNG
BLOOD & LUNGS 34
• It is often associated with systemic lupus erythematosus .
• Pulmonary embolism is the most common lung-associated complication of the
antiphospholipid syndrome and may be the initial clinical event.
• Chronic pulmonary thromboembolism may contribute to pulmonary hypertension .
• The lung is frequently involved, and death may result from hypoxic respiratory
failure.
• Treatment strategies include anticoagulation, plasmapheresis, intravenous immune
globulin, and steroids.
36. MULTIPLE MYELOMA
BLOOD & LUNGS 36
• multiple myeloma is manifested primarily by widespread skeletal destruction
and frequently is associated with anemia, hypercalcemia, and renal
dysfunction.
• Pulmonary manifestations are rare.
• homogeneous mass associated with an osteolytic rib lesion,
• Pulmonary parenchymal involvement by the abnormal plasma cells is
unusual. Diffuse pulmonary infiltration by neoplastic plasma cells
occasionally produces interstitial changes on the chest roentgenograph.
37. MULTIPLE MYELOMA - cont
BLOOD & LUNGS 37
• Unusual manifestations include pleural effusions, chylothorax,
and pulmonary parenchymal calcification.
• Alveolar hemorrhage has been described as a presenting
feature of myeloma.
• Metastatic pulmonary calcification that resolved with therapy is
described in a patient with multiple myeloma.
38. HSTIOCYTIC RETICULOCYTOSIS
BLOOD & LUNGS 38
• Pulmonary Langerhans cell histiocytosis (PLCH) The near universal
association of PLCH with cigarette smoking strongly implies a causative role.
• Patients usually present following a spontaneous pneumothorax or with a
nonproductive cough, dyspnea, or fever or weight loss.
• Recurrent spontaneous pneumothorax occurs in 15 to 25 percent of patients.
• HRCT scan of the chest showing a combination of multiple cysts and
nodules, with a mid to upper zone predominance, and interstitial thickening in
a young smoker is so characteristic
39. HSTIOCYTIC RETICULOCYTOSIS -cont
BLOOD & LUNGS 39
• Pulmonary function tests may be normal or may show reduced lung volumes
and a reduced diffusing capacity. Airflow limitation and hyperinflation occur in
a minority of patients, typically in patients with more advanced, cystic
disease
• If the radiographic pattern is nondiagnostic, the combination of
transbronchial lung biopsy and bronchoalveolar lavage (BAL) showing an
increase in the number of CD-1a positive cells strongly suggests PLCH,
especially when more than 5 percent of BAL cells are CD1a positive
• The main focus of any therapeutic regimen in patients with pulmonary
Langerhans cell histiocytosis is cessation of smoking.
40. AMYLOIDOSIS
BLOOD & LUNGS 40
• rare disease that occurs when a substance called amyloid builds up in your
organs. Amyloid is an abnormal protein that is usually produced in your bone
marrow and can be deposited in any tissue or organ.
• Systemic amyloidosis :
1. The lower respiratory tract is affected in the form of nodular lesions
(solitary or multiple) with cavitations or calcifications.
2. Diffuse alveolar septal form leading to sever decrease in diffusing
capacity, progressive lung disease, sever hypoxemia.
3. Hemoptysis with dissection of medium sized pulmonary arteries,
• Treatment: (colchicine, steroids)
41. AMYLOIDOSIS - cont
BLOOD & LUNGS 41
• localized amyloidosis :
Sleep apneaMacroglossia
Stridor, dyspneaLaryngeal and subglottic (localized, stenotic)
Stridor, dyspnea, hemoptysisDiffuse tracheobronchial (submucosal plaques)
Stridor, dyspnea, hemoptysisLocalized tracheobronchial (masslike lesions)
Mild symptoms, bronchiectasis, cavitation in 30%Diffuse nodular (parenchymal)
Rare, minimal symptoms, incidental findingSolitary nodular (parenchymal, amyloidoma)
Progressive dyspnea, hemoptysisDiffuse parenchymal (septal or interstitial)
Seen in 5% of all amyloidosesMediastinal and hilar lymphadenopathy
43. LEUKEMIA
BLOOD & LUNGS 43
• The reported mortality rate associated with pulmonary
complications in leukemia is approximately 60% to
65%.
• The respiratory complications depend on the type of
leukemia, the nature and course of treatment, and the
presence or absence of significant neutropenia.
44. LEUKEMIA - infections
BLOOD & LUNGS 44
• Many of the complications, particularly infections, are secondary to the
immunocompromised status of leukemic patients, which is caused either by
the leukemic state itself or by treatment.
• Infectious pneumonia is a frequent and often fatal complication and is
responsible for up to 75% of deaths in patients with acute leukemia.
• Gram-negative organisms are the most common cause of pneumonia.
• Fungal pneumonia occurs in up to 30% of patients.
45. LEUKEMIA - infections
BLOOD & LUNGS 45
• Granulocytopenia in leukemic patients poses a significant risk of
invasive aspergillosis and ARDS because invasive pulmonary
aspergillosis is a life-threatening complication.
• Massive hemoptysis may occur in some patients.
• Prognosis has been uniformly poor, with mortality rates
exceeding 70% in some series.
46. LEUKEMIA - pulmonary
parenchyma
BLOOD & LUNGS 46
• lymphocytic leukemia is more likely than the granulocytic type to invade
pulmonary parenchyma.
• diffuse bilateral reticulonodular infiltration resembling that of lymphangitic
metastasis.
• In patients who are granulocytopenic, presence of diffuse lung infiltrates
should warn of the possibility of invasive aspergillosis.
• The leukemic infiltrates may be parenchymal (focal or diffuse), pleural,
peribronchial, or endobronchial.
47. LEUKEMIA - pulmonary alveolar
hemorrhage
BLOOD & LUNGS 47
• Pulmonary hemorrhage usually is associated with
thrombocytopenia and may be extensive.
• Another predisposing cause is invasive pulmonary aspergillosis.
• The majority of patients with pulmonary alveolar hemorrhage do
not exhibit hemoptysis.
48. LEUKEMIA - others
BLOOD & LUNGS 48
• Mediastinal and hilar adenopathy is seen in 50% of cases.
• Leukemic cell lysis pneumopathy, which occurs as a result of
pulmonary vascular occlusion by destroyed leukemic cells within
48 hrs of initiation of chemotherapy, may cause respiratory
failure.
• Bleomycin pulmonary toxicity
50. LEUKEMIA - others
BLOOD & LUNGS 50
• Pulmonary leukostasis
- Hyperleukocytosis denotes peripheral white blood cell count in excess of
50,000/mm3.
Pathogenic mechanisms
• Increased blood viscosity in the microcirculation
• Leukocyte aggregation
• Leukocytic microthrombi
• Release of toxic products from leukemic cells
• Complement activation
• Eendothelial cell damage
• Microvascular invasion
51. LEUKEMIA - others
BLOOD & LUNGS 51
• Patients usually present with new onset shortness of breath or worsening
dyspnea.
• On physical exam, patients are tachypneic, and lung exam reveals diffuse
rales.
• If severe, patients with leukostasis can develop hypoxia, fever and
progressive respiratory acidosis, all of which indicate a worse prognosis.
• No radiological findings have been clearly associated with pulmonary
leukostasis. The chest radiograph may be normal or show an atypical
bilateral reticular pattern with septal lines or bilateral airspace
consolidation
52. LEUKEMIA - others
BLOOD & LUNGS 52
• Pulmonary alveolar phospholipoproteinosis
- lung biopsy may suggest areas of pulmonary alveolar
phospholipoproteinosis.
- This is secondary to the monocytopenia in leukemia.
- Alveolar macrophages are derived from monocytes, and the
deficiency of monocytes in leukemia results in the inability of the
limited number of alveolar macrophages to ingest intra-alveolar
phospholipids.
53. LEUKEMIA - others
BLOOD & LUNGS 53
• Pseudohypoxemia or spurious hypoxemia,
-denotes low oxygen tension and saturation in arterial blood in the absence of
clinical evidence of tissue hypoxia.
- This phenomenon occurs in patients with extreme degrees of leukocytosis.
- During the in vitro transportation of an arterial blood sample from the patient
to the laboratory, the large number of leukocytes in the syringe consume
significant amounts of oxygen, and hence, the measurement reveals a low
PaO2.
- Pseudohypoxemia is seen also in patients with severe thrombocytosis.
54.
55. POLYCYTHEMIA
BLOOD & LUNGS 55
• Defined as a sustained excess of red blood cell volume
• Secondary polycythemia is a compensatory mechanism seen in various
chronic hypoxemic states.
• The pulmonary manifestations in patients with such diseases are those of the
underlying disease.
• Primary polycythemia, or polycythemia rubra vera, is a chronic disease
of unknown etiology characterized by hyperplasia of all the cellular elements
of the bone marrow, nucleated red blood cells being more prominently
involved.
56. POLYCYTHEMIA - cont
BLOOD & LUNGS 56
• Acute airway obstruction caused by spontaneous
retropharyngeal bleeding and hematoma formation has
been described in a patient with polycythemia rubra
vera.
• As the result of increased whole blood viscosity thrombotic
events are common .
• Pulmonary edema.
58. HODGKIN'S DISEASE
BLOOD & LUNGS 58
• Intrathoracic involvement in Hodgkin's disease is common,
occurring in up to 40% of patients.
• Pulmonary involvement may be seen in more than 50% of
cases of Hodgkin's lymphoma at postmortem. Intrathoracic
involvement is twice as common with Hodgkin's as with non-
Hodgkin's lymphoma.
• any type of chest roentgenographic abnormality can be seen
in patients with thoracic manifestation of Hodgkin's disease.
59. HODGKIN'S DISEASE - cont
BLOOD & LUNGS 59
• the most common abnormality is the enlargement of
mediastinal lymph nodes, noted in 50% of cases.
• Pulmonary parenchymal involvement is seen in up to 30% of
patients, especially in those with the nodular sclerosing type
of Hodgkin's disease, and is usually accompanied by
mediastinal lymphadenopathy.
60. HODGKIN'S DISEASE - cont
BLOOD & LUNGS 60
The parenchymal features include:
• direct invasion of lung from regional lymph nodes (characterized by
linear densities),
• massive homogeneous infiltrates with lymphadenopathy,
• nodular infiltrates,
• generalized dissemination resembling miliary tuberculosis.
61. HODGKIN'S DISEASE - cont
BLOOD & LUNGS 61
• Endobronchial involvement occurs in nearly 5% of patients
with Hodgkin's lymphoma.
• Lobar or segmental atelectasis, cough, and hemoptysis may
result.
• Extrinsic compression of the trachea and main stem bronchi by
large mediastinal Hodgkin's lymphoma can lead to airway
obstruction and respiratory failure.
62. HODGKIN'S DISEASE - cont
BLOOD & LUNGS 62
• Pleural effusion occurs in 30% of patients and usually is associated with
other intrathoracic lesions.
- obstruction to lymphatics by the enlarged hilar lymph nodes.
-The pleural fluid is commonly an exudate, serous, and chylous in one-
third.
- Massive pleural effusions have occurred as a late complication of radiation
therapy for Hodgkin's lymphoma, probably from impaired lymphatic drainage
secondary to mediastinal fibrosis induced by radiation.
• Spontaneous pneumothorax is an unusual complication in Hodgkin's
disease.
63. NON-HODGKIN'S LYMPHOMAS
BLOOD & LUNGS 63
• Mediastinal lymph node enlargement
-It is the most common intrathoracic manifestation of non-Hodgkin's
lymphoma, seen in nearly 35% of patients.
• Primary pulmonary lymphoma ordinarily presents as an alveolar infiltrate or
a homogeneous mass.
• Bronchial obstruction or endobronchial involvement occurs, but less
frequently than with Hodgkin's lymphoma.
64. NON-HODGKIN'S LYMPHOMAS - cont
BLOOD & LUNGS 64
• solitary or multiple nodules 3 mm to several centimeters in diameter, more
frequently in the lower lobes.
• Pleural effusions are common in non-Hodgkin's lymphoma.
• Pleural effusion in patients with non-Hodgkin's lymphoma is usually
secondary to pleural lymphoma rather than to obstruction of mediastinal
lymphatics.
- Systemic chemotherapy results in resolution of pleural effusion in
approximately half the patients; the prognosis is poor in those with refractory
effusions.
65. LYMPHOMATOID GRANULOMATOSIS
BLOOD & LUNGS 65
• Recently renamed angiocentric T-cell lymphoma, polymorphic reticulosis, midline
malignant reticulosis, midline granuloma, and Stewart's granuloma.
• clinically and roentgenologically mimics Wegener's granulomatosis
• middle age, and there is a slightly higher prevalence in men.
• non-specific and include fever, malaise, and weight loss.
• can affect any organ system, but the disease is found with greatest frequency in the
CNS, skin, kidney, and lymphatic system.
•
66. LYMPHOMATOID GRANULOMATOSIS -cont
BLOOD & LUNGS 66
• 1. CNS:
- Nearly one-fourth of patients .
- Ataxia, hemiparesis, blindness, and dizziness are the presenting symptoms.
• 2. SKIN:
- Almost half of patients .
- erythematous, macular, or plaque-like lesions over the extremities.
67. BLOOD & LUNGS 67
• 3. PULMONARY DISEASE
- Pulmonary involvement is present in virtually all patients with
lymphomatoid granulomatosis.
- Along with the systemic symptoms, cough and dyspnea are
prominent respiratory symptoms.
- If head and neck areas are involved, patients may present with
symptoms similar to those of Wegener's granulomatosis.
- Hemoptysis is more likely in those with cavitated lung
lesions.
- Airway involvement is unusual but can be extensive.
68. BLOOD & LUNGS 68
• Chest roentgenograms
• Most frequently disclose nodular infiltrates. Nodular densities
may cavitate and are more common in the lower lung zones.
• Occasionally, alveolar infiltrates are noted.
• Pleural effusions occur in 25% of patients.
• Presence of hilar or mediastinal lymphadenopathy .
• Unilateral or bilateral large pulmonary masses measuring more
than 10 cm in diameter often signal the presence of lymphoma.
69. LYMPHOMATOID GRANULOMATOSIS -cont
BLOOD & LUNGS 69
• The diagnosis of lymphomatoid granulomatosis requires biopsy examination
of the affected tissue,
• Lung biopsy characteristically shows an angiocentric angiodestructive
infiltration of atypical lymphocytoid and plasmacytoid cells.
• Treatment and Prognosis
- There is no definitive therapy for lymphomatoid granulomatosis.
- Multiple chemotherapeutic agents with a corticosteroid may be needed in
patients who demonstrate highly malignant features.
- Localized lesions in the head and neck area may respond to radiation.
70. POEMS SYNDROME
BLOOD & LUNGS 70
• polyneuropathy, organomegaly, endocrinopathy, and skin
changes syndrome, also known as Crow-Fukase syndrome.
• rare variant of plasma cell dyscrasia with multisystemic
manifestations.
• Markedly elevated levels of vascular endothelial growth factor
were responsible for the acute arterial obliteration described in
several patients.
• Pulmonary hypertension has been described in this syndrome.
71. CASTLEMAN'S DISEASE
BLOOD & LUNGS 71
• Castleman's disease (CD, angiofollicular lymph node hyperplasia) is a
lymphoproliferative disorder associated with infection from the human
immunodeficiency virus (HIV) and human herpesvirus .
• age between 50 and 65 with fever, night sweats, weight loss, and weakness
or fatigue. Peripheral generalized lymphadenopathy , and often accompanied
by hepatosplenomegaly. Laboratory abnormalities include anemia,
hypoalbuminemia, hypergammaglobulinemia, and an elevated sedimentation
rate.
• Almost all treatments using single agents (eg, anti-viral, anti-cytokine,
chemotherapy, corticosteroids) are palliative, with disease recurrence once
they are stopped.
72. LYMPHOCYTIC INTERSTITIAL
PNEUMONITIS
BLOOD & LUNGS 72
• characterized by pulmonary parenchymal infiltrates that consist
predominantly of small lymphocytes and variable numbers of
plasma cells
• Many patients with lymphocytic interstitial pneumonitis have
developed lymphomas. Indeed, all cases of lymphocytic
interstitial pneumonitis are thought to represent low-grade
lymphomas.
74. THROMBOCYTOPENIA
BLOOD & LUNGS 74
• Thrombocytopenia is defined as a platelet count less than 150,000/microL
(150 x 10(9)/L), keeping in mind that 2.5 percent of the normal population will
have a platelet count lower than this .
Pulmonary manifestations
• Pulmonary hematoma
• Hemothorax
• Fatal pulmonary hemorrhage
• ITP is associated with DIC and venous thromboembolism
75. THROMBOCYTOSIS
BLOOD & LUNGS 75
• Thrombocytosis is defined as a platelet count >500,000/microL
Causes :
• Reactive thrombocytosis (RT)
• Essential thrombocythemia (ET)
• Regardless of cause, a high platelet count has the potential to be associated
with vasomotor (headache, visual symptoms, light headedness, atypical
chest pain,, erythromelalgia), thrombotic, or bleeding complications
80. TRANSFUSION AND THE LUNG
BLOOD & LUNGS 80
The use of blood and blood products, even under the best
circumstances, carries considerable risk for the recipient.
- Immediate pulmonary reactions include:
• dyspnea,
• bronchospasm,
• pulmonary edema
81. TRANSFUSION-RELATED ACUTE
LUNG INJURY
BLOOD & LUNGS 81
• new acute lung injury (ALI)/acute respiratory distress syndrome
(ARDS) occurring during or within six hours after blood product
administration .
• When a clear temporal relationship to an alternative risk factor
for ALI/ARDS coexists, a formal diagnosis of TRALI cannot be
made. In these circumstances, the diagnostic terminology to be
used is ―possible TRALI.‖
82. BLOOD & LUNGS 82
Recipient risk factors
• Liver transplantation
• surgery
• Shock
• Higher peak airway pressure while being mechanically
ventilated
• Current smoking
• Positive fluid balance
Transfusion-Related Acute Lung Injury - cont
83. BLOOD & LUNGS 83
• all blood components have been associated with TRALI. This
includes transfused whole blood ,derived platelets,, and
granulocytes , as well as intravenous immunoglobulin
preparations , and allogeneic stem cells.
• signs and symptoms associated with non-cardiogenic pulmonary
edema Hypoxemia: in an intubated patient this could manifest
as a change in oxygenation or increased oxygen requirements .
Transfusion-Related Acute Lung Injury - cont
84. BLOOD & LUNGS 84
• Pulmonary infiltrates on chest radiography .
• If previously intubated, pink frothy airway secretions from the
endotracheal tube .
• Fever .
• Hypotension .
• Cyanosis .
Transfusion-Related Acute Lung Injury - cont
85. BLOOD & LUNGS 85
DIFFERENTIAL DIAGNOSIS: —
• Transfusion-associated circulatory overload (TACO) .
• risk factors for TACO include compromised cardiac function ,
positive fluid balance, and rapid blood product administration.
Transfusion-Related Acute Lung Injury - cont
86. TRANSFUSION-RELATED ACUTE LUNG INJURY -cont
BLOOD & LUNGS 86
• Management of the patient with TRALI/possible TRALI is
supportive, with oxygen supplementation for the correction of
hypoxemia being the cornerstone of treatment.
• Non-invasive respiratory support with (CPAP) or (BiPAP) may be
sufficient in less severe cases, but endotracheal intubation with
invasive mechanical ventilation is often required
• Other complications of blood transfusion like overload , sepsis ,
anaphylaxis , bronchospasm .
88. POSTPERFUSION SYNDROME
BLOOD & LUNGS 88
• Pulmonary complications following prolonged cardiopulmonary bypass .
• The etiology remains unknown, although immunologic mechanisms have
been suggested.
• After prolonged maintenance on cardiopulmonary bypass, patients develop
progressive pulmonary insufficiency, which is manifested by cyanosis,
hypoxemia, increased work of breathing as a result of severely diminished
compliance, and widening of the alveolar–arterial oxygen tension
gradient.
89. POST-PERFUSION SYNDROME -cont
BLOOD & LUNGS 89
• Chest roentgenograms reveal patchy, diffuse alveolar infiltrates that resemble
pulmonary edema.
- Factors that contribute to the postperfusion lung syndrome include:
• hypoxia,
• interruption of blood supply to pulmonary tissues ,
• an underlying pulmonary disease process.
• Prevention of the postperfusion syndrome is important because treatment is not
promising.
• Corticosteroids given early may help.
91. MISCELLANEOUS
BLOOD & LUNGS 91
• Paroxysmal nocturnal hemoglobinuria
- hematopoietic stem cell disorder characterized by an increased sensitivity
of blood cells to complement-mediated lysis.
- Thrombosis of the pulmonary vasculature and pulmonary hypertension
have been described.
• Hematopoiesis
- Acute and rapidly fatal respiratory failure resulting from pulmonary interstitial
extramedullary hematopoiesis associated with myelofibrosis has been
described.
92. BLOOD & LUNGS 92
IMPACT OF
PULMONARY
DISEASES ON
BLOOD
93. IMPACT OF PULMONARY DISEASES
ON BLOOD
BLOOD & LUNGS 93
On red blood cells :
• Secondery Polythycemia due to hypoxemia .
• Anemia due to chronic diseases like TB .
• Falling HB level in pulmonary alveolar hemorrhage .
On platelets :
• Thrombocytosis in severe infections .
• Thrombocytopenia due to disseminated intravascular
coagulation (DIC) , antiphospholipid syndrome , and drug
induced like HIT , Lineozolid .
94. IMPACT OF PULMONARY DISEASES
ON BLOOD - cont
BLOOD & LUNGS 94
Thrombocytopenia in the ICU patient
• Infection, sepsis, septic shock
• Massive blood transfusion
• Cardiopulmonary resuscitation
• Adult respiratory distress syndrome
• Pulmonary embolism
• Use of intravascular catheters
95. IMPACT OF PULMONARY DISEASES ON
BLOOD - cont
BLOOD & LUNGS 95
On White blood cells :
• leucocytosis due to infections .
• Leukopenia in severe cases .
• Lymphocytosis in viral pneumonia and sometimes TB.
• Lymphopenia as in steroid therapy .
• leukomoid reaction in severe infections.
• Eosinophilia in Asthma , Churg Strass synderome , and Fungal
infections like Aspergillosis.
96. BLOOD & LUNGS 96
Others :
• Drug induced Bone marrow depression like
chemotherapy .
• DIC .
IMPACT OF PULMONARY DISEASES ON
BLOOD - cont