Aplastic Anemia

Carbamazepine may lead to aplastic anemia.

Ref: Harrisons principles of internal medicine, 18 th edition, Page: 3263, Table: 369/9

Ans. is ‘d’ i.e., Parvo virus B-19

“Parvovirus B-19 induces aplastic crisis in children with chronic hemolytic anemias, as in sickle cell disease”. Parvovirus

.                                   Non-enveloped DNA (ss DNA) viruses.

.                                   Only DNA virus which has single stranded DNA.

.                                   Smallest virus

.                                   Has smallest genome

.                                   Only one medically important human parvovirus – Parvovirus B 19

.                                   Transmission is by respiratory route and through blood.

.                                   Infection is commonly acquired in childhood (5-10 yrs).

.                                   Most of the infections are asymptomatic.

.                                   Most common presentation is respiratory infection with an erythematous maculopapular rash and arthralgia – Erythema infectiosum or Fifth disease.

.                                   Starts with prominant erythema of cheeks –> slapped cheek disease.

.                                   Parvovirus B-19 induces aplastic crisis in children with chronic hemolytic anemias, as in sickle cell disease.

.                                   Parvovirus B-19 infection during second or third trimester of pregnancy may result in nonimmune fetal hydrops.

Ans. is ‘a’ i.e., Aplastic anaemia; ‘b’ i.e., MDS; ‘c’ i.e., Megaloblastic anaemia

Ans. is ‘c’ i.e., Reticulocytopenia

Aplastic anemia

o Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. o Aplastic anemia patients have lower counts of all the three blood cells types : ‑

Red blood cells          —>         Anemia and Reticulocytopenia

White blood cells -4                  Leukopenia (Neutropenia)

Platelets                      —>         Thrombocytopenia

o This type affection of all type of blood cells is also known as Pancytopenia.

“Aplastic anemia is pancytopenia with bone marrow hypocellulalarity-.

• Splenomegaly is characteristically absent; if present, the diagnosis of aplastic anemia should be ruled out.

Ans. is ‘a’ i.e., AML; ‘b’ i.e., Myelodysplastic anemia; ‘c’ i.e., Paroxysmal nocturnal hemoglobinuria

Aplastic anemia can progress to : –

Paroxysmal nocturnal hemoglobinuria, Myelodysplastic anaemia, Rarely acute leukemia.

Ans. is ‘c’ i.e., Pure red cell aplasia

Ans. is All

Answer is A (Parvovirus)

Aplastic crisis is usually triggered by an acute parvovirus infection.

Answer is D (Increased LAP scores)

PNH is associated with a decreased Leukocyte Alkaline phosphase (LAP) score

PNH: Paroxysmal Nuctunal haemoglobinuria: Review

Answer is D (Cold haemoglobinuria)

Paroxysmal Cold Hemoglobinuria is a form of immune mediated hemolytic anemia and is not a form of aplastic anemia.

Pregnancy, PNH and hepatitis have all been mentioned as causes of Aplastic anemia.

Classification Of Aplastic Anemia And Cytopenias :

Acquired Aplastic Anemia                                                                                      Inherited Aplastic Anemia

Answer is C (Hepatitis A)

Aplastic anemia is associated with Non-A, Non-B, Non-C, Hepatitis and not with Hepatitis A Parvovirus B19, HIV-1, Chloramphenicol and PNH are all associated with Aplastic anemia.

Answer is C (Aplastic anemia)

Patient in question has anemia with pancytopenia and an enlarged spleen. Presence of an enlarged spleen makes aplastic anemia the least likely diagnosis. All other conditions mentioned as options may present with both pancytopenia and splenomegaly.

• Aplastic anemia  may present with anemia and pancytopenia but not with an associated enlarged spleen. If splenomegaly is present the diagnosis of aplastic anemia is seriously questioned.’ – Robbins
• Megaloblastic anemia: Besides ineffective synthesis and increased hemolytic destruction of red blood cells, there also occurs premature destruction of granulocytes and platelet resulting in Leucopenia and thrombocytopenia -Pancytopenia (Robbins) An enlarged spleen may be associated.
• Leukemia :Aleukemic form of Acute Lymphocytic leukemia also explains both – pancytopenia and hepatosplenomegaly
• A chronic disease associated with splenomegaly also explains pancytopenia as a sequels to increased destruction as a manifestation of 1-1YPERSPLENISM’ (increased sequestration and destruction of cells in sinusoids of an enlarged spleen).

Answer is A (Splenomegaly):

Splenomegaly is characteristically absent in Aplastic Anemia

‘Splenomegaly is characteristicall^y absent. If present, the diagnosis of aplastic anemia should he seriously questioned’

Answer is A (Antithymocyte globulin followed by cyclosporine)

Tor adults over age 50 years or those without HLA matched siblings, the treatment of choice for severe Aplastic anemia is immunosupression with antithymocyte globulin (A TG) plus Cvclosporine’

Answer is D (Gall stone disease)

Gallstone disease has not been mentioned in the list for extrahepatic manifestations of Hepatitis B

Extrahepatic manifestations in viral hepatitis B:

• Aplastic Anemia
• Cryoglobulinaemia
• Diabetes mellitus
• Dermatomyositis-like syndrome
• Gianotti- Crosti syndrome
• Glomerulonephritis
• Gui!lain- Barre syndrome
• Myasthenia gravis
• Pericarditis and myocarditis
• Pancreatitis
• Peripheral Neuropathy
• Polyarleritis Nodosa
• Polyarthritis
• Polymyalgia rheumatica
• Polymyositis
• Porphyria cutanea tarda
• Psychosis
• Raynaud’s syndrome
• Serum sickness like illness
• Splenomegaly and lymph node enlargement
• Skin alterations
• Thrombopenic^. purpura
• Thyroiditis
• Urticaria

Ans. C: Hepatocellular carcinoma

Patients usually have dysmorphic features or physical stigmata. On occasion, marrow failure may be the initial presenting feature.

– Fanconi anemia

Dyskeratosis congenita

Cartilage-hair hypoplasia

Pearson syndrome

Amegakaryocytic thrombocytopenia

– Familial aplastic anemia

Acquired causes of aplastic anemia (80%)

Idiopathic factors

Infectious causes, such as hepatitis viruses, EBV, human immunodeficiency virus (HIV), parvovirus, and mycobacteria

Toxic exposure to radiation and chemicals, such as benzene

Drugs and elements, such as chloramphenicol, phenylbutazone, and gold may cause aplasia of the marrow. PNH is caused by an acquired genetic defect limited to the stem-cell compartment affecting the PIGA gene. Mutations in the PIGA gene render cells of hematopoietic origin sensitive to increased complement lysis.

Ans. A i.e. Aplastic anemia

Aplastic anemia

• Causes:

—        Drugs (chloramphenicol),

—        Viruses etc.

• Feature absent in aplastic anemia: Splenomegaly
• Most effective treatment of aplastic anemia: Bone marrow transplant

Ans. is ‘a’ i.e., Exposure to drugs